PMID- 31505044
OWN - NLM
STAT- MEDLINE
DCOM- 20200914
LR  - 20210110
IS  - 1365-2265 (Electronic)
IS  - 0300-0664 (Linking)
VI  - 91
IP  - 6
DP  - 2019 Dec
TI  - What is the appropriate management of nonfunctioning pancreatic neuroendocrine 
      tumours disclosed on screening in adult patients with multiple endocrine 
      neoplasia type 1?
PG  - 708-715
LID - 10.1111/cen.14094 [doi]
AB  - Multiple endocrine neoplasia type 1 (MEN1) is an inherited tumour syndrome 
      characterised by a predisposition to the development of endocrine tumours of the 
      parathyroid glands, pituitary and pancreas: 30%-80% of patients with MEN1 develop 
      pancreatic neuroendocrine tumours (pNETs), with metastatic tumours and/or their 
      sequelae contributing to increased morbidity and early mortality. The optimal 
      management of nonfunctioning (NF) pNETs in MEN1 remains controversial. Whilst 
      pancreatic resection is widely recommended for tumours >2 cm, for smaller tumours 
      (</=2 cm) a well-established consensus guiding the indications for surgical 
      intervention does not exist. Although total pancreatectomy may be curative for 
      some patients, both short- and long-term complications make this an 
      unsatisfactory option for many patients. For small (<2 cm) MEN1 NF-pNETs, some 
      clinicians advocate surveillance based largely on retrospective data that suggest 
      50%-80% of these lesions are stable over time and infrequently exhibit 
      accelerated growth rates. It is increasingly recognised, however, that NF-pNETs 
      exhibit unpredictable malignant behaviour that is not determined by tumour size 
      alone, thereby prompting other clinicians to advocate surgery for all MEN1 
      NF-pNETs, irrespective of size. Such uncertainty poses clinical management 
      challenges with regards to the timing and extent of surgery, which is further 
      hindered by the inability to stratify patients based on predicted tumour 
      behaviour. It is therefore critical that future MEN1 research initiatives 
      include: (a) the discovery of biomarkers that better predict tumour behaviour; 
      (b) the evaluation of medical therapies that may delay, or even prevent, the need 
      for pancreatic surgery; and, ultimately, (c) improvement in the quality of life 
      for individuals with MEN1. Here, based on the published literature, we address 
      the Clinical Question, 'What is the management of NF-pNETs disclosed on screening 
      in adult patients with MEN1?'.
CI  - (c) 2019 John Wiley & Sons Ltd.
FAU - Challis, Benjamin G
AU  - Challis BG
AUID- ORCID: 0000-0002-1130-2851
AD  - Wolfson Diabetes and Endocrinology Clinic, Cambridge University Hospital NHS 
      Foundation Trust, Cambridge, UK.
AD  - Translational Science & Experimental Medicine, Research and Early Development, 
      Cardiovascular, Renal and Metabolism, BioPharmaceuticals R&D, AstraZeneca, 
      Cambridge, UK.
FAU - Casey, Ruth T
AU  - Casey RT
AUID- ORCID: 0000-0003-4058-3135
AD  - Wolfson Diabetes and Endocrinology Clinic, Cambridge University Hospital NHS 
      Foundation Trust, Cambridge, UK.
AD  - Department of Medical Genetics, Cambridge University, Cambridge, UK.
FAU - Grossman, Ashley
AU  - Grossman A
AD  - Oxford Centre for Diabetes, Endocrinology and Metabolism, University of Oxford, 
      Oxford, UK.
AD  - Royal Free Hospital ENETS Centre of Excellence, London, UK.
AD  - Centre for Endocrinology, Barts and the London School of Medicine, London, UK.
FAU - Newell-Price, John
AU  - Newell-Price J
AD  - Department of Oncology and Metabolism, The Medical School, University of 
      Sheffield, Sheffield, UK.
FAU - Newey, Paul
AU  - Newey P
AUID- ORCID: 0000-0003-4414-0278
AD  - Division of Molecular & Clinical Medicine, Ninewells Hospital & Medical School, 
      University of Dundee, Dundee, Scotland.
FAU - Thakker, Rajesh V
AU  - Thakker RV
AD  - Academic Endocrine Unit, Radcliffe Department of Medicine, University of Oxford, 
      Oxford, UK.
LA  - eng
GR  - SCAF/15/01/CSO_/Chief Scientist Office/United Kingdom
PT  - Journal Article
PT  - Review
DEP - 20191001
PL  - England
TA  - Clin Endocrinol (Oxf)
JT  - Clinical endocrinology
JID - 0346653
RN  - Non functioning pancreatic endocrine tumor
SB  - IM
MH  - Disease Progression
MH  - Female
MH  - Humans
MH  - Male
MH  - Multiple Endocrine Neoplasia Type 1/*complications/surgery
MH  - Neuroendocrine Tumors/diagnosis/etiology/surgery
MH  - Pancreatic Neoplasms/complications/diagnosis/*etiology/surgery
OTO - NOTNLM
OT  - chromogranins < Hormones/related: <Neuroendocrine tumours
OT  - computed tomography < Investigations & Rx: <Adrenal
OT  - indium-111-octreotide scintigraphy < Investigations & Rx: <Neuroendocrine tumours
OT  - multiple endocrine neoplasia type 1 < Conditions: <Genetic
EDAT- 2019/09/11 06:00
MHDA- 2020/09/15 06:00
CRDT- 2019/09/11 06:00
PHST- 2019/07/15 00:00 [received]
PHST- 2019/09/04 00:00 [revised]
PHST- 2019/09/05 00:00 [accepted]
PHST- 2019/09/11 06:00 [pubmed]
PHST- 2020/09/15 06:00 [medline]
PHST- 2019/09/11 06:00 [entrez]
AID - 10.1111/cen.14094 [doi]
PST - ppublish
SO  - Clin Endocrinol (Oxf). 2019 Dec;91(6):708-715. doi: 10.1111/cen.14094. Epub 2019 
      Oct 1.